O' Connor Timothy, McNally Cora, Kennedy Mark W
Cardiology Department, Beaumont Hospital, Beaumont road, Dublin D09V2N0, Ireland.
Infectious Diseases Department, Beaumont Hospital, Beaumont road, Dublin D09V2N0, Ireland.
Eur Heart J Case Rep. 2023 Aug 18;7(9):ytad397. doi: 10.1093/ehjcr/ytad397. eCollection 2023 Sep.
Kawasaki disease (KD) is an acute systemic vasculitis which predominantly occurs in childhood but rarely in adulthood. Diagnosis relies on the presence of typical clinical features; however, patients may present atypically, increasing the challenge of timely diagnosis for physicians.
We report a case of a 40-year-old male presenting with persistent fever, rash, and unilateral neck swelling. Initial investigations were suggestive of necrotizing lymphadenitis, with a presumed infective aetiology. However, extensive microbiology and immunological investigations remained negative. Cardiac injury was evident with elevated troponin T and NT-proBNP; however, left ventricular systolic function was normal. After 4 days, clinical features consistent with KD were noted and the results of a lymph node biopsy supported this diagnosis. Despite timely treatment with intravenous immunoglobulins (IVIG) and high-dose aspirin, follow-up computed tomography (CT) coronary angiography demonstrated two sequential aneurysms (max 6 mm) in the right coronary artery, plus one small subtle aneurysm in the proximal left anterior descending artery (4 mm).
Diagnosis of adult KD remains challenging, as symptoms often present sequentially over time rather than simultaneously and many of the clinical features necessary for diagnosis share commonality with other infectious disease processes.
川崎病(KD)是一种急性全身性血管炎,主要发生于儿童期,在成人中罕见。诊断依赖于典型临床特征的存在;然而,患者可能表现不典型,这增加了医生及时诊断的挑战。
我们报告一例40岁男性患者,表现为持续发热、皮疹和单侧颈部肿胀。初始检查提示坏死性淋巴结炎,推测病因是感染。然而,广泛的微生物学和免疫学检查均为阴性。心肌损伤明显,肌钙蛋白T和N末端脑钠肽前体(NT-proBNP)升高;然而,左心室收缩功能正常。4天后,出现了与川崎病一致的临床特征,淋巴结活检结果支持这一诊断。尽管及时给予静脉注射免疫球蛋白(IVIG)和高剂量阿司匹林治疗,但随访计算机断层扫描(CT)冠状动脉造影显示右冠状动脉有两个连续动脉瘤(最大6毫米),左前降支近端还有一个小的隐匿性动脉瘤(4毫米)。
成人川崎病的诊断仍然具有挑战性,因为症状往往随时间依次出现而非同时出现,且诊断所需的许多临床特征与其他感染性疾病过程有共同之处。